AB0638 INTRAVENOUS IMMUNOGLOBULIN IN ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS. STUDY OF 28 CASES FROM A SINGLE UNIVERISTARY HOSPITAL AND LITERATURE REVIEW
Authors: F. Benavides-Villanueva, C. Corrales-Selaya, J. Loricera, V. Calvo-Río, S. Castañeda, N. Aviles Mendez, J. M. Blanco Madrigal, M. A. González-Gay, R. Blanco
Journal: Annals of the Rheumatic Diseases (2022)

Background Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) includes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) or microscopic polyarteritis (MPA). Standard treatment is often accompained by significant adverse events. Intravenous immunoglobulins (IVIG) may constitute a therapeutic alternative, however, the data are scarce. Objectives To assess the utility and safety of IVIG in AAV. Methods Observational study of patients with AAV from Spanish referral center treated with IVIG. AAV diagnosis was based on a compatible clinical presentation and/or positive ANCA serology and/or histology. Disease activity was assessed with the Birmingham Vasculitis Activity Score (BVAS). Results We included a total of 28 patients; GPA (n=15), MPA (10), and EGPA (3). The main features are summarized in Table 1. The reasons for using IVIG were: a ) relapse/refractory disease (n=20), or presence/suspicion of infection (8). We observed a rapid and maintained Clinical improvement, since first month of IVIG onset, yielding a BVAS score of zero in 56.5% of patients at 24 months (Figure 1). Serious Adverse event was only observed in 1 patient who developed congestive cardiac failure and had to stop the IVIG therapy. Table 1. Main general features of 28 patients with antineutrophil cytoplasmic antibody-associated vasculitis treated with intravenous immunoglobulins. GENERAL FEATURES RESULTS GENERAL FEATURES (Continuation) RESULTS (Continuation) DEMOGRAPHIC FEATURES ANALITICAL FINDINGS Age of Diagnosis of AAV, mean±SD 57.1±18 CRP (mg/dL), median [IQR] 13.02 Men/ Women; n, (% men) 15/13 (53.6%) ESR, mm/1 st hour, median [IQR] 70.4 AAV Subtype, n (%) PR3-ANCA, n (%) 11 (39.3) GPA 15(53.6%) MPO-ANCA, n (%) 12 (42.8) EGPA 3(10.7%) ANCA negative, n(%) 5 (17.8) MPA 10(35.7%) FFS at AAV diagnosis, n (%) CLINICAL MANIFESTATIONS, n (%) 0 10 (35.7) Fever 15 (53.6%) 1 11 (39.3) Constitutional symptoms 26 (92.85%) 2 7 (25) ORL involvement 7 (25%) PREVIOUS TREATMENT, n (%) Pulmonary involvement 19 (67.9%) Cyclophospamide 13 (46.4%) Renal involvement 25 (89.3%) Methotrexate 6 (21.4%) Cutaneous involvement) 6 (21.5%) Azathioprine 3 (10.7%) Ocular involvement 4 (14.3%) Cyclophosphamide 13 (46.4%) Joint involvement 4 (14.3%) Mycophenolate mofetil 4 (14.3%) Neurologic involvement 8 (28.57%) Rituximab 5 (17.9%) Abbreviations: ANCA:antineutrophil cytoplasmic antibody; EGPA: eosinophilic granulomatosis with polyangiitis; FFS: Five-Factors Score; GPA: granulomatosis with polyangiitis; MPA: microscopic polyangiitis Figure 1. BVAS Evolution with IVIG treatment of all our patients. Conclusion IVIG seems to be an effectiveness and relative safe therapeutic option in relapse/refractory AAV or in presence of a concomitant infection. Disclosure of Interests None declared